Prognostic factors for relapse and survival among patients with ocular adnexal lymphoma: validation of the eighth edition of the American Joint Committee on Cancer (AJCC) TNM classification.

BACKGROUND/AIMS: To validate the prognostic performance of the American Joint Committee on Cancer (AJCC) eighth edition classification for ocular adnexal lymphoma (OAL). METHODS: We performed a retrospective review of 140 consecutive patients treated for primary OAL between March 2010 and September 2017. Associations between T/N/M categories at presentation and disease-related outcomes, including relapse, progression-free survival (PFS) and overall survival (OS) were evaluated. RESULTS: Seventy-nine women and 61 men (median age, 52 (range 20-84) years; median follow-up, 57 (range 7-131) months) were included. Histological subtypes included mucosa-associated lymphoid tissue lymphoma (92.1%, n=129), diffuse large B-cell lymphoma (5.0%, n=7), follicular lymphoma (1.4%, n=2) and mantle cell lymphoma (1.4%, n=2). Patients with ≥T2 disease had significantly higher risks of overall relapse (unadjusted HR)=4.32, p=0.016), decreased PFS (uHR=5.19, p=0.004) and decreased OS (uHR=9.21, p=0.047). Patients with ≥N1 disease had significantly higher risks of overall relapse (uHR=9.17, p<0.001) and decreased PFS (uHR=9.24, p<0.001). M1 disease was significantly associated with higher risks of overall relapse (uHR=3.62, p=0.036), decreased PFS (uHR=5.13, p=0.001) and decreased OS (uHR=9.24, p=0.013). On considering TNM categories as continuous data, the uHRs for per level increase in T, N and M categories were 1.77, 1.83 and 2.30 for overall relapse and 1.72, 1.87 and 2.78 for decreased PFS, respectively (p<0.05 for each comparison). CONCLUSION: The T, N and M categories of the AJCC eighth edition classification have prognostic value for relapse and survival among patients with primary OAL. Particularly, nodal/metastatic involvement at presentation indicated less favourable outcome.

Postoncological lacrimal duct reconstruction: A practical classification system for reconstructive planning and short-term results of a case series.

BACKGROUND: Surgical resection of skin tumors in the medial canthal area may damage the lacrimal duct and can result in chronic epiphora. Postoncologic reconstruction of the lacrimal duct has not been studied extensively. The current study discusses the anatomical and functional features of the lacrimal duct. It describes short-term functional outcomes after monocanalicular reconstruction of the lacrimal duct in a case series of 10 patients. METHODS: From February 2015 to October 2017, all patients with a postoncological lacrimal duct defect were analyzed to make an anatomical classification. The functional outcomes of patients after monocanalicular reconstruction were measured with the Munk scale up to 3 months after stent removal. RESULTS: Twelve patients had lacrimal duct defects after Mohs resection. Anatomical characteristics were used to create a clinical classification for lacrimal duct defects. This classification divides the upper (U) and lower (L) proximal lacrimal duct into two sections which can be damaged: the punctum and pars verticalis (1), the canaliculus horizontalis (2), or combined (3). The Common lacrimal duct (C) is the distal part of the lacrimal duct and can also be affected. Ten patients were analyzed after lacrimal duct reconstruction. Three months after stent removal, none of the patients suffered from epiphora. CONCLUSIONS: This article proposes an anatomical classification for lacrimal duct defects in the proximal lacrimal drainage system. The classification can be applied in comparing cases and determining reconstructive strategies after oncologic skin tumor resection. Short-term results are promising for future efforts to reconstruct the lacrimal duct.

Lacrimal Sac Tumors--A Review.

Tumors of the lacrimal sac are rare but their recognition and early management are imperative, as they are locally invasive and potentially life-threatening. Because of their rarity, large clinical studies with statistically significant data on the natural course, management, and prognosis of these neoplasms are unavailable. Current practices are therefore based on a few case series and a small number of isolated case reports. Most tumors are primary and of epithelial origin (60-94%), of which 55% are malignant. Lacrimal sac tumors typically present with epiphora and a palpable mass over the medial canthus and are thus often erroneously diagnosed as chronic dacryocystitis. A full history with clinical and diagnostic workup is essential to plan treatment, which is often multi-disciplinary. Statistically significant associations have been shown with higher tumor staging and size with increased metastatic risk and lower survival rates. Management usually involves complete surgical resection with adjuvant radiotherapy and/or chemotherapy for malignant lesions. Long-term follow-up is required, as recurrences and metastases can occur many years after initial treatment.

An Update on Tumors of the Lacrimal Gland.

Lacrimal gland tumors are rare and constitute a wide spectrum of different entities ranging from benign epithelial and lymphoid lesions to high-grade carcinomas, lymphomas, and sarcomas with large differences in prognosis and clinical management. The symptoms and findings of a lacrimal gland lesion are a growing mass at the site of the lacrimal gland, including displacement of the eyeball, decreased motility, diplopia, and ptosis. Pain is the cardinal symptom of an adenoid cystic carcinoma. Radiological findings characteristically include an oval, well-demarcated mass for benign lesions whereas malignant lesions typically display calcifications, destruction of bone, and invasion of adjacent structures. The diagnosis ultimately relies on histology, as does the choice of treatment and the prognosis. In recent years, the understanding of the biology of numerous types of lacrimal gland neoplasia has improved and the choice of treatment has changed accordingly and holds further promise for future targeted therapies. Treatment of benign epithelial lesions is surgical excision whereas carcinomas often require adjuvant radiotherapy and/or chemotherapy. In contrast, the cornerstone in management of lymphoid lesions is chemotherapy, often including a monoclonal antibody. This article presents an update on the clinical, radiological, histological, and molecular features, along with treatment strategies for tumors of the lacrimal gland.

The Dacryocystorhinostomy Ostium Granulomas: Classification, Indications for Treatment, Management Modalities and Outcomes.

AIM: Ostium granulomas following dacryocystorhinostomy (DCR) have not been studied in detail previously. This study aims to classify the DCR-related granulomas based on their ostial locations and to assess the outcomes of their management. METHODS: A retrospective consecutive case series of 47 ostial granulomas evaluated over a period of 2 years were included in this study. All patients underwent detailed endoscopic examination to assess the granuloma locations and their response to initial topical steroids treatment. Persistent granulomas either underwent further management with excision or intralesional steroids based on their location. Patients were followed up for a minimum of 6 months. The primary outcome measure was resolution of granuloma. RESULTS: The mean age at presentation was 45 years with a female preponderance (68%). 70% (33/47) of granulomas were following external DCR and 30% (14/47) occurred in the setting of endonasal DCR. The most common location was an edge granuloma in 46.8% (22/47) followed by a combined granuloma in 21.2% (10/47). 91.4% (43/47) underwent initial treatment with topical nasal steroids. The remaining 4 (8.5%) underwent primary excision. Among those treated with topical steroids (n = 43), 9.3% (4/43) underwent further treatment with intralesional triamcinolone. Overall, 4.2% (2/47) recurred in 6 weeks following resolution and were treated with excision. CONCLUSION: We recommend routine endoscopic evaluation of all the DCR ostia. Detection of granulomas in early stages and appropriate management as per guidelines proposed may aid in better outcomes.

Review of 268 lacrimal gland biopsies in an Australian cohort.

BACKGROUND: To review the distribution of pathology in lacrimal gland biopsies performed in an Australian cohort. DESIGN: Retrospective review. PARTICIPANTS: Two hundred sixty-eight lacrimal gland biopsies from 263 patients. METHODS: Pathology archives in South Australia and Victoria were searched for lacrimal gland biopsies performed between 1 January 1997 and 31 December 2012. Data retrieved included the year of biopsy, the histopathological diagnosis, patient age and gender. MAIN OUTCOME MEASURES: Distribution of pathology affecting the lacrimal gland; patient age and gender. RESULTS: The distribution of lacrimal gland pathology was: inflammations and vasculitides 50.0%, lymphomas 19.8%, lymphoid hyperplasias 12.3%, benign epithelial tumours 7.8% (all pleomorphic adenomas), malignant epithelial tumours 4.1%, dacryops 3.0% and miscellaneous 3.0%. The mean age was 52 years, with lymphoma affecting the oldest patient group (64.6 years) and sarcoidosis the youngest (40.6 years). Of the patients with biopsy-confirmed dacryoadenitis, biopsy revealed a specific diagnosis in 34% of cases. Immunoglobulin G4-related disease was the most common 'specific' dacryoadenitis. Significantly more pleomorphic adenomas were diagnosed in the period 1997-2004 than the period 2005-2012 inclusive, but there were no other significant changes in the distribution of pathology over time. CONCLUSIONS: Two thirds of dacryoadenitis was 'non-specific', two thirds of epithelial tumours were pleomorphic adenomas and approximately two thirds of all lacrimal gland biopsies were accounted for by inflammations and lymphoid hyperplasias. The ratio of inflammations to neoplasias will be significantly influenced by the clinician's threshold for biopsying patients presenting with features of dacryoadenitis.

[Single-photon emission computed tomography in combination with X-ray computed tomography in the study of lacrimal passages. First results].

OBJECTIVE: To explore the capabilities of single-photon emission computed tomography (SPECT) in combination with x-ray computed tomography (SPECT/CT) in the study of the anatomy and drainage function of lacrimal passages. MATERIAL AND METHODS: Twelve scans were carried out in 6 healthy volunteers and in patients with different lacrimal drainage disorders (dacryostenoses, varying obstructive lacrimal passage, dacryocystitis and post-dacryocystorhinostomy). Sodium pertechnetate (99mTc) solution and a radiocontrast agent were instilled into the conjunctival sac during the study. RESULTS: SPECT is an objective procedure to study lacrimal drainage function. It cannot provide data on the anatomic structure of lacrimal passages and determine the location of their stenosis or obstruction. SPECT/CT makes it possible to simultaneously obtain information on the anatomic structure of lacrimal passages, to visualize the sites of their pathological changes, and to evaluate the drainage function of lacrimal passages.

Lacrimal gland lesions in Denmark between 1974 and 2007.

PURPOSE: To evaluate the incidence rate, distribution, patient characteristics and indications for surgical intervention of lacrimal gland lesions in Denmark between 1974 and 2007. MATERIAL AND METHODS: All biopsied/surgically removed lacrimal gland lesions in Denmark during the period 1974-2007 were identified by searching two population-based registries. Specimens were collected and re-evaluated. The following data were collected: age, gender, indications for surgical intervention and local recurrence. RESULTS: A total of 232 lesions from 210 patients with a histologically verified lesion of the lacrimal gland were included. The incidence rate of lacrimal gland lesions was 1.3/1 000 000/year. The overall annual age- and gender-adjusted incidence rate more than doubled during the study period, owing to an increase in non-malignant lesions. Approximately half of the lesions were neoplasms (119) and 55% (66) of these were malignant. Dacryops constituted 10% (24), inflammatory lesions 27% (62), normal tissue 12% (27), benign tumours 23% (53) and malignant tumours 29% (66). Patients with malignant neoplasms were significantly older than patients with benign neoplasms (63 versus 48 years, p < 0.001). The indication for surgical intervention was suspicion of a tumour in more than 90% of the neoplastic lesions and in 30% of the non-neoplastic lesions. CONCLUSION: Lacrimal gland lesions that require surgical evaluation are rare in the Danish population and represent a wide spectrum of diagnoses, mostly benign. The overall incidence rate of biopsied lacrimal gland lesions is increasing.

Perspective: what does the term functional mean in the context of epiphora?

The term 'functional block' is used to encompass the concept of lacrimal drainage dysfunction in the presence of anatomical patency. There is significant variability in the nomenclature, clinical and investigative criteria used in the literature to define this entity. This has led to confusion and lack of comparability of studies looking at this group. The clinical features, investigative findings and treatment options in this group of patients are described. In addition, the need to differentiate between nasolacrimal stenosis and 'functional block' is emphasized, as evidence suggest patients with nasolacrimal duct stenosis have better outcomes with dacryocystorhinostomy. To better define the disease entity and enable comparability of future studies, it was proposed that this entity be replaced by the term functional epiphora with no delay, pre- or post-sac delay.

Tumors of the lacrimal drainage system.

Tumors of the lacrimal drainage system are rare, but potentially life-threatening. They comprise a large and variable spectrum of entities grouped into three major categories of primary epithelial, primary nonepithelial and inflammatory lesions. The most common primary epithelial tumors include papilloma, squamous cell carcinoma and transitional cell carcinoma, the most frequent primary nonepithelial tumors fibrous histiocytoma, malignant lymphoma and malignant melanoma, and the most common inflammatory lesions sarcoidosis, Wegener granulomatosis and pyogenic granuloma. This review outlines the incidence, types, management and prognosis of tumors affecting the lacrimal drainage system.

[Classification of orbital tumors]. / Classification des tumeurs orbitaires.

Knowledge of orbital tumor classification is essential because it is the basis of an optimal therapeutic strategy. These tumors comprise the sphenoorbital meningiomas, the optic nerve sheath meningiomas, the optic nerve gliomas, the schwannomas, the histiocytic tumors, the metastases, the lacrimal gland tumors, the mesenchymal tumors of the soft tissues, the primary orbital wall tumors, the tumors arising from the nasal and paranasal sinuses, the hematopoietic tumors, the vascular tumors, and the congenital tumors.

Epithelial lacrimal gland tumors: pathologic classification and current understanding.

OBJECTIVE: To apply the updated epithelial salivary gland classification scheme to a large cohort of lacrimal gland tumors so as to provide an updated lacrimal gland tumor classification scheme. METHODS: A retrospective multicenter cohort study of 118 cases of epithelial neoplasia was undertaken. Main outcome measures included pathologic analysis, subtyping, and survival. RESULTS: Of 118 cases, 17 (14%) were reclassified using the proposed expanded classification scheme based on the current World Health Organization classification of salivary gland tumors. The most frequent neoplasms were pleomorphic adenoma and adenoid cystic carcinoma, of which we highlight more unusual histologic features. Three tumors were found to be unclassifiable with the updated scheme, with 2 having histologically malignant features. Deficiencies and variations in pathologic assessment were noted. Variation in the histologic findings of pleomorphic adenoma and assessment of the extent of invasion of carcinoma ex pleomorphic adenoma were highlighted. CONCLUSIONS: The use of the more histologically diverse classification of salivary gland tumors can be successfully applied to the epithelial lacrimal gland neoplasms. This expanded classification system led to reclassifying 14% of cases. Currently, there are no consistent pathologic standards for processing and evaluating these lesions.

American Joint Committee on Cancer classification predicts outcome of patients with lacrimal gland adenoid cystic carcinoma.

PURPOSE: To investigate whether American Joint Committee on Cancer (AJCC) classification at initial diagnosis of lacrimal gland adenoid cystic carcinoma predicts outcome of treatment on local recurrence. DESIGN: Retrospective chart review. PARTICIPANTS: Consecutive patients with adenoid cystic carcinoma of the lacrimal gland treated at 8 institutions between January 1986 and December 2007. METHODS: Clinical records, including pathology reports and imaging studies, were reviewed. MAIN OUTCOME MEASURES: AJCC classification, histologic subtype, local recurrence rate, and survival. RESULTS: AJCC classification at initial diagnosis was assessable for 53 patients and was as follows: T1N0M0, 7 patients; T2N0M0, 8 patients; T3aN0M0, 14 patients; T3aNxM0, 1 patient; T3aN0M1, 1 patient; T3bN0M0, 13 patients; T3bN0M1, 1 patient; T4aN0M0, 2 patients; T4bN0M0, 4 patients; T4bN0M1, 1 patient; and T4bNXM0, 1 patient. Thirty-eight (72%) of the 53 patients had >T3 tumors at presentation. Of the 38 patients with >T3 tumors, 20 were treated with orbital exenteration and postoperative adjuvant radiotherapy (RT), 6 were treated with orbital exenteration without RT, and 12 were treated with globe-preserving surgery (10 with RT and 2 without RT). Of the 15 patients with T3 tumors, the risk of local recurrence (in the orbit or skull base) was higher in patients treated with conservative surgery as opposed to orbital exenteration and RT. Only 4 (20%) of the 20 patients treated with orbital exenteration and RT had local recurrence, compared with 3 (50%) of the 6 patients treated with orbital exenteration without RT and 8 (67%) of the 12 patients treated with globe-preserving surgery. Overall, 17 (45%) of the 38 patients with >T3 tumors and only 1 (7%) of the 15 patients with T3 disease at initial diagnosis correlates with worse outcomes than does AJCC

Epithelial tumors of the lacrimal gland: an update.

PURPOSE OF REVIEW: The goal of this article is to offer an update on the treatment and prognosis of the most common epithelial tumors of the lacrimal gland, report on new pathological entities and offer a review of the classification of lacrimal gland tumors. RECENT FINDINGS: Improvements have been made in the understanding of lacrimal gland lesions with the knowledge that lacrimal gland tumors compare to the more common counterparts of the major salivary glands. Therefore, the WHO's classification of salivary gland tumors has been adapted to the lacrimal gland pathology. Until recently, primary adenocarcinomas of the lacrimal gland were not further subclassified, but they can now be divided into low-grade and high-grade malignancies. The adjunctive use of intra-arterial cytoreductive chemotherapy for the management of adenoid cystic carcinoma is one of the most important advancements on the management of these aggressive tumors. Another important step forward has been taken on carcinoma ex pleomorphic adenoma of the lacrimal gland, which is subclassified into noninvasive carcinoma, with an excellent prognosis after complete excision and invasive carcinoma for which the prognosis is still guarded despite adjunctive radiotherapy. SUMMARY: This article offers an update on diagnosis, classification and treatment of common and rare epithelial lacrimal gland tumors.

[Classification of ocular surface disease. Part 1]. / Klassifikation der Erkrankungen der Augenoberfläche. Teil I.

The ocular surface consists of the lid margin, conjunctiva and cornea which together with the tear system represent a functional entity. The diagnosis of ocular surface disease can be very difficult due to the similarity of various disease entities. The classification should be made on the pathological and pathophysiological characteristics of ocular surface disease. The first part of the classification comprises diseases of the lid margin, the tear system as well as diseases of the conjunctiva. Both the clinical presentation as well as the underlying pathophysiological and pathological characteristics of the most important ocular surface diseases are reviewed.

Three different types of congenital lacrimal sac fistulas.

Three different symptoms in 4 patients with congenital lacrimal sac fistulas are presented. The first symptom was epiphora since birth, the second symptom was infection of the lower eyelid, and the third symptom was tearing from the eye in a 76-year-old patient. This patient had nasolacrimal obstruction at the canal level and no symptoms of congenital lacrimal sac fistula. She had undergone excision, including dacryocystorhinostomy. Three of the 4 patients underwent excision of the fistulous tract. The fistula originated from the lacrimal sac in all patients. Symptomatic congenital lacrimal sac fistulas can be treated successfully with excision alone or with excision and dacryocystorhinostomy in cases of nasolacrimal obstruction.